IMEX   05356
INSTITUTO DE MEDICINA EXPERIMENTAL
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Answer to Aenosine Diphosphato-Epinephrine (ADP-EPI) synergisms in patients with primary deficiency of secretion (PSD)
Autor/es:
DOMÍNGUEZ MP; ALBERTO MF; BERMEJO EI; MESCHENGIESER SS; SÁNCHEZ-LUCEROS A; LAZZARI MA
Lugar:
Tel-Aviv, Israel
Reunión:
Congreso; 19th International Congress on Thrombosis-The Mediterranean League against Thromboembolic Diseases; 2006
Institución organizadora:
The Mediterranean League against Thromboembolic Diseases
Resumen:
ANSWER TO ADENOSINE DIPHOSPHATO -EPINEPHRINE (ADP-EPI) SYNERGISMS IN PATIENTS WITH PRIMARY DEFICIENCY OF SECRETION (PSD)) M.P. Domínguez, MF. Alberto. E. Bermejo. S.S. Meschengieser.A. Sánchez Luceros. M.A. Lazzari flemostasia y Trombosis, Instituto de Investigaciones Hematológicas.  Academia Nacional De Medicina DL’ Buenos Aires. Bueno.s Aires, Argentina PSD is a group of patients with reduced ATP release and normal primarv aggregation, storage pon1 and arachidonic acid (AA) metabolisms. This group may represent heterozygous P2YI2 abnorrnality expression. In plateleis with  P2YI2 deficiency. ADP is unable to inhibit adenylyl cyclase (AC) through  Gei2 protein; this pathway can be restored using Epi in synergic ssav.  Objective: Study the answer to ADP-Epi synergisms. measuring ATP release  hy bioluminescence, in PSD with mucocutaneous bleeding. Methods:  bleeding time (IVY), APTT, FVIII, VWF:Ag, VWR:RCo. platelet  aggregation and ATP release using: 2.5-lOuM ADP, 10 uM Epi. l-8ug/ml  Collagen (Col), 0.5mM AA and ADP-Epi with: ( 0.625-3.0) uM. (1.250 - 3.0) uM. (2.500- 10) uM respectively. Results: ln agreement with plateleo  aggregation three phenotypes were defined: A) without secondary  aggregation for ADP and Epi (n12), B) without secondary aggregation only  for ADP (n=9), C) normal (n=87). Aggregation and secretion with AA and Col were normal in all groups. There were no correlation between the  phenotypes and bleeding severity (p>O.3). neither with vWF parameters  (p>0.3). The values of ATP (uM) released were expressed in the table.  Conclusion: Secondary aggregation deficiency was corrected by AC  inhibition through the synergic effect of Epi, showing a functional alteration  of P2Yl2. Besides, the distinct anssser between A and B phenotypes using  ADP-Epi would suggest differences al molecular structure. Citation: Pathophysiol Haemost Thromb 2006; 35: Abst 1095